In which of the following conditions, Aniridia and Hemihypertrophy are most likely present -
High-Yield Explanation
Wilm's tumour is associated with three groups of congenital malformations.
The risk of Wilm's tumour is increased in these conditions.
These are -
1. WAGR syndrome - It consists of
Aniridia
Mental retardation
Genital anomalies
The risk of Wilm's tumour is increased by 33% in this syndrome.
2. Denys-Drash syndrome - It consists of
Gonadal dysgenesis (male pseudohermaphroditism)
Nephropathy leading to renal failure.
Majority of patients with this syndrome have a ranal failure.
3. Beckwith - Wiedemann syndrome - It consists of
Enlargement of body organs
Hemihypertrophy
Renal medullary cysts.
Abnormal large cells in the adrenal cortex.